Sudden Onset Auditory Neuropathy Spectrum Disorder: A Rare Case Report of Brown Vialetto Van Laere Syndrome

Indian J Otolaryngol Head Neck Surg. 2024 Feb;76(1):1187-1190. doi: 10.1007/s12070-023-04207-0. Epub 2023 Sep 11.

Abstract

Brown Vialetto Van Laere syndrome is a rare disorder characterized by progressive pontobulbar palsy with bilateral sensorineural hearing loss and lower cranial nerve palsies. Fifty-eight cases have been reported in the last hundred years. As the most common presenting complaint of this disorder is hearing loss, audiological evaluation plays a vital role in pointing towards and narrowing its diagnosis. We present a case report of a 12-year male child affected by this disorder.